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Education in Heart
Congenital heart disease in adult patients
Cardiopulmonary exercise testing and sports participation in adults with congenital heart disease
  1. Jonathan Buber1,
  2. Keri Shafer2,3
  1. 1 Department of Medicine, Division of Cardiology, University of Washington School of Medicine, Seattle, Washington, USA
  2. 2 Department of Cardiology, Boston Children’s Hospital, Boston, Massachusetts, USA
  3. 3 Division of Cardiology, Brigham and Women’s Hospital, Boston, MA, United Startes of America
  1. Correspondence to Dr Jonathan Buber, University of Washington Medical Center, Seattle, WA 98195, USA; bubery{at}u.washington.edu

https://doi.org/10.1136/heartjnl-2018-313928

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    Learning objectives

    • Describe the key role of objective evaluation of exercise capacity as part of the routine clinical follow-up in adult congenital heart disease (ACHD) patients.

    • List-specific advantages of cardiopulmonary exercise testing in ACHD patients and recognise the unique exercise patterns associated with common congenital heart defects.

    • Outline possible advantages of routine physical activity across the spectrum ACHD patients while recognising potential caveats for specific lesions.

    Patient presentation

    Malcolm is a 35-year-old patient followed at a safety net hospital. He describes growing up with limited resources both in his family and at his school. As the child of a single mother, his afternoons were often spent hanging out in his neighbourhood. He recalls that there were two options ‘basketball or gangs’. Because of his complex congenital heart disease, he was not allowed to play sports and his exercise capacity was never evaluated. Now, 20 years later, he feels that was a turning point in his life which ultimately resulted in a 5-year incarceration for weapons charges. ‘I loved basketball and not playing made me angry and feel different. I made my own choices, but playing would have made a huge difference’. Malcolm has his life back on track now, but providers have a responsibility to ensure that kids like him have providers who consider their whole lives and the lifelong impact of their recommendations. Restriction is not only a physical limitation. It affects patient’s self-perception, acceptance and psyche. Thus, providers must consider the risks of not playing sports versus that of playing, and recommend level of activity based on objectively obtained data.

    Introduction

    The benefits of exercise are well known to most patients and providers. Due to exercise restrictions placed of patients with congenital heart disease (CHD) as well as concerns for exercise-related morbidity and mortality, many providers and patients are uncertain on ways to maximise fitness at minimal risk for their CHD patients,1 and the number of adult congenital heart disease (ACHD) patients advised to engage in an exercise routine or who receive a formal referral to cardiac rehabilitation remains low.2 3 Current data indicate that >90% of children born with CHD in high-resource countries survive to adulthood and there is a 5% annual growth rate of the ACHD population.4 5 As some of the most important benefits of exercise are seen in all adults, it is imperative that ACHD patients and their providers are well educated on exercise strategies.6

    In general, most patients with ACHD can safely engage in regular, physical activity of at least moderate intensity,7 yet some high-risk conditions, such as ventricular dysfunction, severe outflow obstruction, large aortic aneurysms and pulmonary arterial hypertension require a more tailored set of recommendations. Exercise recommendations to ACHD patients are based on a comprehensive clinical assessment that often includes routine exercise testing. Cardiopulmonary exercise testing (CPET) is the suitable evaluating ACHD patients as it is capable of demonstrating exercise capacity and be used to determine the mechanism of exercise limitations. CPET was therefore endorsed by the American Heart Association/American College of Cardiology/ACHD guidelines for both baseline functional assessment and serial testing for ACHD patients.8

    This review will focus on these two main topics, namely CPET and sports prescription and participation across the spectrum of the ACHD population.

    Exercise testing in ACHD

    Exercise intolerance is virtually universal in patients with CHD and it impairs their quality of life. Furthermore, the rate of deterioration of peak oxygen consumption (VO2) (the highest rate of VO2 detected during a progressive exercise test) which is estimated at ~0.3%–0.7%/year after age 21 years,9 is more rapid in complex forms of CHD.10 Despite having objectively reduced exercise capacity, many ACHD patients report ‘normal’ functional status to their physicians, as their perception of normal can be distorted.11 This can also limit the provider’s perception of exercise intolerance, and underlies the importance of obtaining routine objective assessment of exercise capacity in ACHD patients, preferably via CPET.8 12

    Extracardiac causes to exercise intolerance in ACHD

    The ability to perform sustained exercise depends on appropriate performance of the cardiovascular, repsiratory and musculoskeletal systems. As it is common for for ACHD patients to have multiorgan dysfunction, when assessing exercise intolerance, common extracardiac aetiologies for exercise limition such as lung disease, anaemia and inadequate muscular oxygen utilisation/uptake should be considered.

    Lung disease

    Lung disease is common in ACHD patients: approximately one-third patients have moderate to severe abnormalities on spirometry. The most common abnormality is restrictive lung disease with low forced vital capacity (FVC) and total lung capacity. Causes of restrictive lung disease include hypoplastic lung growth (eg, Scimitar syndrome), previous thoracotomies/sternotomies, diaphragmatic paralysis due to surgical phrenic nerve injury, abnormal chest wall mechanics (eg, scoliosis, pectus excavatum), enlarged cardiac structures and obesity.13 14 Lesions that restrict pulmonary blood flow such as pulmonary valve stenosis or tetralogy of Fallot (ToF) can limit lung growth and also lead to restrictive lung disease. Obstructive lung disease is also common. The aetiology of obstructive lung disease in ACHD is less clear but may include concomitant airway/vascular malformations (such as decreased total alveoli volume or even extension of smooth muscle tissue into the pulmonary vasculature associated with complex ventricular septal defect/conotruncal lesions), airway compression due to vascular structures (eg, vascular ring) and medications such as non-selective beta blockers.15 16 Congestive heart failure leads to diminished gas exchange, decreased diaphragmatic function and reduced lung compliance.

    Shunts can damage the pulmonary vasculature; lesions that involve left to right shunting (particularly large ventricular septal defects or patent ductus arteriosus) lead to pulmonary over-circulation and vascular remodelling and can cause pulmonary arterial hypertension.

    Anaemia and erythrocytosis

    Anaemia results in reduced oxygen carrying capacity and a premature shift to predominant anaerobic metabolism (lower anaerobic threshold during exercise) and can worsen symptoms and exercise capacity in patients with heart failure.17 Anaemia is relatively common in ACHD patients and can be multifactorial in origin, including ineffective erythropoiesis, poor iron absorption, bleeding from anticoagulation therapy, haemolysis from prosthetic valves, kidney disease and anaemia of chronic disease. Cyanotic patients with secondary erythrocytosis may have hyperviscosity, avid oxygen binding and impaired oxygen delivery; however, these patients rarely undergo CPET. Given that ACHD patients may be particularly sensitive to effects of anaemia and that erythrocytosis may cause hyperviscosity and alter blood flow, evaluation should be pursued in those with unexplained or significant exercise intolerance. There are currently no clear recommendation to check blood counts prior to CPETs, and further investigation is needed.

    Skeletal muscle abnormalities

    Skeletal muscle abnormalities which impair oxygen extraction will also cause the peak VO2 to be depressed. Important causes of skeletal muscle abnormalities include glycogen storage diseases and mitochondrial or other metabolic defects. It is important to consider these defects in patient with genetic syndromes that have multiorgan effects (eg, Turner syndrome).18 Severe and long-standing deconditioning leads to a reduction in the normal augmentation of preload and stroke volume (SV) by pumping action of the exercising skeletal muscles,9 and ACHD patients (as well as heart failure patients) may be particularly sensitive to the decreased skeletal muscle efficiency with deconditioning.3

    CPET interpretation in ACHD

    Cardiopulmoanry exercise testing can be performed with either treadmill or cycle ergometer. Individuals who exercise on a treadmill reach 5%–10% higher peak VO2 and anaerobic threshold values. In pacemaker-dependent patients, rate responsiveness algorithms may be better assessed on a treadmill than cycle.

    The principal considerations for CPET interpretation are delineated in table 1. Below, we provide a more CHD-directed outline for CPET interpretation.

    View this table:
    Table 1

    Select parameters evaluated at cardiopulmonary exercise tests

    Peak VO2

    The Peak VO2 is the most important indicator of cardiopulmonary function in patients with CHD as it correlates with cardiac output response.19 Vo2 is normalised to age, body weight and gender.

    Not surprisingly, patients with CHD have impaired maximal VO2 compared with age-matched controls free of heart disease. Therefore, it can be useful to compare maximal VO2 to patients to similar forms of CHD to determine whether a patient has impairment beyond what would be expected based on their congenital abnormality. Figure 1 shows reference values derived from a large ACHD cohort of patients with various defect types.20 Although much of these data are based on an older cohort of patients and may not reflect average exercise capacity in a more contemporary population, it remains the single source for reference values in ACHD CPETs to date.

    Figure 1
    Figure 1

    Peak VO2 data expressed as percentage of predicted value. The density lines above the histogram and the numbers to the right of the graph relate to all patients with a given diagnosis. The numbers above the density lines indicate percentage peak VO2 values for the 10th, 25th, 50th, 75th and 90th centiles. ASD, atrial septal defect; ccTGA, congenitally corrected TGA; CoA, coarctation of aorta; complex, complex congenital heart disease (including univentricular hearts); Ebstein, Ebstein anomaly; Eisenmenger, Eisenmenger syndrome; Fontan, patients after Fontan palliation; TGA, transposition of the great arteries; ToF, tetralogy of Fallot; VO2, oxygen consumption; Valvular, mixed collective of patients with congenital valvular heart disease; VSD, ventricular septal defect. Reprinted by permission from reference 20.

    As can be appreciated from figure 1, there is a good correlation between the degree of exercise intolerance and severity of the underlying cardiac lesion or repair. Patients with the most complex lesions such as complex cyanotic heart defects or Eisenmenger’s syndrome typically have the lowest peak VO2 values and greatest degree of ventilatory inefficiency. A similar correlation exists between the temporal rate of deterioration of peak VO2 and defect severity. The types and outcomes of prior interventions performed for the congenital defect may also bear substantial effects on exercise capacity and on CPET results. For example, patients with a right ventricle (RV) serving as the chamber supporting the systemic circulation (‘systemic RV’) have worse exercise capacity as compared with those with a systemic left ventricle (LV, figure 2).20 21 Also, patients with residual valve stenosis or regurgitation,22 23 residual shunts24 or those who underwent a baffle creation or conduit implantation and have residual baffle obstruction or a leak25 were all shown to have diminished exercise capacities.

    Figure 2
    Figure 2

    Theoretical graphical CPET displays of two types of surgeries for D-loop transposition of the great arteries. Left: a patient who is s/p atrial switch operation: intraatrial baffles redirect systemic venous return to the mitral valve and LV, pulmonary venous return to the tricuspid valve and the right ventricle serves as the systemic pumping chamber. Peak VO2 values (bottom left image) are markedly reduced at about 11 mL/min/kg and the VE/VCO2 slope is markedly elevated at 45 (bottom second from left image, normal: up to 30). Right: a patients who is s/p arterial switch operation: reimplantation of the great vessels at their correct anatomic location, with restoration of the left ventricle as the systemic pumping chamber. Peak VO2 values are nearly normal at 25 mL/min/kg (bottom right image) and the VE/VCO2 slope is normal at 28. CPET, cardiopulmonary exercise testing; LV, left ventricle; VCO2, carbon dioxide production;  VE, minute ventilation; VO2, oxygen consumption.

    Despite limitations, utilisation of the published standards in ACHD patients20 can be useful in normalising exercise response. Values that are <15.5 mL/kg/min have been shown to correlate with worse overall prognosis in ACHD patients.21 For patients with Fontan circulation—who lack a subpulmonary ventricle—augmentation of cardiac output during exercise is mainly achieved due to the work of the lower extremity muscles, with the largest increase in stroke volume achieved during initial stages (free wheeling).26 The role of the so-called ‘ventilatory pump’ (the diaphragmatic movement and the creation of negative intrathoracic pressures in stroke volume augmentation) is less clear, and may have variable contribution in the Fontan patients as some studies show minimal additional contribution of this effect on top of the ‘muscle pump’26 while others suggesting a prominent role in the resting cardiac output.27

    VE/ VCO2 slope

    Minute ventilation (VE) rises linearly with carbon dioxide production (VCO2) during progressive exercise until ventilatory threshold when the accumulating lactic acid causes a compensatory increase in VE due to an increase in VCO2. The slope of this relationship (VE/VCO2) is often referred to as ‘ventilatory efficiency’. A steeper slope suggests that a higher VE is required to adequately remove CO2. Aetiologies for the lower ventilatory efficiency include respiratory muscle fatigue, inadequate muscle perfusion (with an earlier anaerobic threshold) are well described in heart failure patients.28 When there is pulmonary blood flow maldistribution either due to pulmonary hypertension or pulmonary oedema, consequent ventilation/perfusion (V/Q) mismatch occurs and ventilatory efficiency is compromised. Additionally right to left shunts (intracardiac or intrapulmonary) also cause the VE/VCO2 slope to be elevated as CO2 rich systemic venous blood to enter the systemic arterial circulation bypassing the lungs. The consequent increase in arterial pCO2 is sensed by arterial chemoreceptors, inducing central nervous system respiratory centres to increase the patient’s respiratory drive (and VE) and causing the VE/ VCO2 slope to rise. Eliminating right to left shunting almost always produces a reduction in the VE/VCO2 slope. In patients with a Fontan palliation, the VE/ VCO2 slope is often significantly elevated due to pulmonary blood flow maldistribution secondary to the absence of a pulmonary ventricle and due to gravity-dependent blood flow.

    Chronotropic response

    Low heart rate (HR) reserve, defined as an inability to increase HR to >80% predicted at peak exercise, or >62% predicted for patients treated with chronotropic agents, is common in the ACHD patients, with an estimated incidence of 60%.29 Chronotropic incompetence may be due to intrinsic malfunction of the conduction system or iatrogenic. In patients with more advanced forms of heart failure, chronic sympathetic overactivity seen leads to β receptor downregulation and reduced myocardial sensitivity to endogenous β agonists, which may in turn lead to a reduction in HR response to exercise. Iatrogenic causes include surgical or catheter based injury to the sinus or atrioventricular nodes or drug therapy. Additionally, those with chronic pacing have abnormal HR increase with exercise as no pacing algorithm perfectly meets the physiological needs of the patient. There are little data to suggest that various degrees of chronotropic incompetence have different prognostic implications.

    Oxygen pulse

    The oxygen pulse (O2P) is calculated by dividing the peak VO2 by the peak HR and correlates closely with forward stroke volume. In patients with low arterial O2 content (ie, patients with anaemia or patients with significant arterial desaturation), O2 extraction will be lower and the O2P may therefore underestimate stroke volume. Erythrocytosis causes the opposite effect, increasing arterial O2 content and causing the O2P to overestimate the stroke volume. Yet as mentioned above, blood testing to evaluate haematocrit levels are not routinely performed for ACHD patients with cyanotic lesions and secondary erythocytosis prior to CPETs, but could be useful in these patients. Patients with impaired ability to augment stroke volume (SV) at peak exercise typically have lower O2P, including those with decreased ventricular function, severe obstructive lesions, severe valvular regurgitation and pulmonary or peripheral vascular disease.30

    Ventilatory anaerobic threshold

    The anaerobic threshold occurs during progressive exercise when aerobic metabolism is insufficient to meet the energy requirements of the exercising muscles, and it is a physiological phenomenon that is less affected by patient effort or motivation. Ventilatory anaerobic threshold (VT) can be determined on a submaximal exercise test and is considered an excellent index of the cardiovascular system’s capacity to support the haemodynamic demands of exercise. The VT tends to be disproportionately depressed in conditions which impair blood flow to the exercising muscles, such as significant systemic ventricular outflow tract obstructions (ie, valve stenosis, aortic coarctation and so on) or with peripheral vascular disease.

    Prognostic implications

    The incorporation of exercise parameters into the medical management plan and long-term prognosis prediction algorithms are topics of ongoing and extensive investigation in the field of ACHD. Although the strength of the data may be limited by the small size of the cohorts and occasionally by the retrospective and non-randomised nature of the studies, CPET results are increasingly used for these purposes when sufficient data exist. For example, in patients with ToF, CPET may be used to assist in guiding the timing of pulmonary valve replacement for severe valve regurgitation, as lower peak VO2 (<20 mL/kg/min) were shown to be associated with higher rates with post-pulmonary valve replacement mortality.31 For patients with D-loop transposition of great arteries (TGA) after an atrial switch operation, the gradual decrease in the systemic RV function was shown to correlate almost linearly with the decrease in peak VO2.32 In patients with the Fontan circulation, a yearly decline in % predicted peak VO2 was a strong predictor of 5-year risk of death or cardiac surgery.33 For patients with Ebstein anomaly, the peak VO2 and HR reserve were significant predictors of adverse cardiac outcomes.34 In one study comparing contemporary age-matched controls with either Fontan or ToF, reduced forced expiratory volume in one second and FVC correlated most strongly with mortality in Fontan patients while VE/VCO2 slope correlated with mortality for ToF patients.35

    Exercise and sports participation in ACHD

    The cardiovascular benefits of exercise proven both in the general population and those with CHD. Although there are extensive data to suggest that sports restriction and limitation of exercise should be avoided if possible, many physicians, patients and families continue to have concerns regarding the risk of arrhythmia or sudden death during vigorous exercise or competitive sports in ACHD patients. Even when athletic restrictions are imposed, there is often discrepancy between the parent, patient, provider and medical record as to which activities are restricted.36

    It is critical to distinguish between physical activity, exercise and competitive sports when considering the need for restriction in patients with CHD. In the vast majority of patients with CHD physical activity and exercise should be promoted, not restricted. There are scant data that there is a relationship between sudden death and regular exercise in patients with CHD; the majority of sudden death in these patients occurs at rest.

    Sudden death/arrhythmia

    For the majority of patients with CHD, the risk of sudden death during exercise or sports is low. Maron et al 1 examined 1866 sudden death events out of an estimated 10.7 million athletes; 690 were as a result of a confirmed cardiac cause. Excluding hypertrophic cardiomyopathy or anomalous coronary arteries, only eight had CHD. While this low rate of ACHD death may be due to a decreased amount of sports participation, several studies suggest that CHD patients’ participation in sport activities is not uncommon. Opic found that 50% of ACHD patients participated in sports with the vast majority in high dynamic sports (eg, basketball, running).37 Other studies also report a relatively high prevalence of sport participation in ACHD patients, although less frequent than the general population.38 39 In all these studies, complex CHD patients were less likely to participate in sports than those with simple defects, yet a significant portion (~47%) participated in sports more strenuous than recommended based on their diagnosis. Importantly, no association with arrhythmias (including premature ventricular beats) or sudden death was observed.

    In a study conducted by Koyak et al, the authors reviewed the cause of sudden death in CHD patients and reported that the occurrence of arrhythmias during exercise testing was associated with sudden cardiac death (SCD).40 However, of the 171 SCDs, only 10% occurred during exercise. In addition, the authors reported a correlation between the type and severity of the congenital defect and the occurrence of SCD, as SCD most commonly occurred in patients with Eisenmenger syndrome, TGA (both D-loop and L-loop) and ToF.

    With regards to implantable cardioverter defibrillator (ICD) implanted patients who participate in sports activities, the data are relatively scarce. When Lampert and colleagues reviewed their ICD registry, ACHD patients were not identified to be at particular risk.41 While there were 46 appropriate shocks, no cardiac deaths occurred in that study. Interestingly, there was no difference in arrhythmic events requiring therapy between competitions, practice and non-sports related physical activity highlighting that these activities likely have similar risk.

    Congestive heart failure/quality of life

    Exercise does not appear to worsen or precipitate heart failure in patients with CHD. In randomised controlled trials of exercise training in CHD, there was no increased risk for decline in ventricular function or heart failure exacerbation, even in high-risk populations with complex CHD.42 43 Moreover, a stabilisation of ventricular function has been found with sports participation,37 suggesting the safety and potential benefit of exercise training even in complex lesions.

    The effect of sports participation on quality of life is inconsistent between studies: Dulfer et al reported a dramatic improvement in cognitive function with sports participation in an children and young adults with CHD, particularly for those with low quality of life,44 whereas Opic et al did not note a difference in quality of life based on sports participation.37

    Sports participation guidelines/recommendations

    In contrast with physical activity and recreational exercise—which is broadly safe in the majority of patients with CHD—many patients with CHD are restricted from certain competitive sports. In 2015, the ACC/AHA guidelines regarding sports participation with CHD were revised.7 Due to a lack of evidence, the 2015 guidelines committee removed excessive restriction of sports participation in favour of less stringent, individualised recommendations that are provided in detail on table 2. The original classification system, which categorised sports as intensity in terms of dynamic and static effort, was retained (figure 3). Both practice/training and competition activities are included in the qualification/disqualification evaluation. For example, collegiate golf teams (class 1A) may include running (class 1C) and weight lifting (class IIIA) as part of the training regimen. Thus, assessment of participation in the sport may not be limited to competition play risk.

    Figure 3
    Figure 3

    Competitive sports classification, based on peak static and dynamic components achieved during competition. Adapted from referrence #7.

    View this table:
    Table 2

    Recommendations for sports participation for adults with congenital heart disease*

    The predominant diagnoses that remain restricted from the majority of sports include those who have severe systemic ventricular dysfunction, significant pulmonary hypertension, severe aortic/pulmonic stenosis, significant cyanosis or ventricular arrhythmias. For athletes with mild to moderate dysfunction or moderately complex CHD (such as ToF), exercise testing is recommended, and if significant arrhythmias or abnormal haemodynamic response are found, limitation to low intensity (class IA) is recommended. When evaluating single ventricle/Fontan patients for sports participation, a personalised approach is recommended including full exercise assessment, ideally with a CPET and modality similar to the desired sport.

    As is the case for consulting all other patients, on consulting on competitive sports participation providers must consider the legal and ethical consequences of their decisions. The provider’s first responsibility is to the patient’s safety, ensuring that the recommendations of qualification/disqualification represent the standard of care. Additionally, providers should strive for clear documentation and communication with the patient. For those acting as team physicians, additional responsibilities to informing the team regarding the safety of play may exist.45

    Exercise for the ACHD patient

    Given that the vast majority of data suggest that exercise prescription is both safe and beneficial in ACHD patients, providers have a responsibility to discuss as well as make recommendations for exercise activity. A complimentary set of guidelines for exercise prescription creation to the existing recommendations for sports participation in ACHD patients discussed above, is not available. In a single expert opinion paper, Budst et al suggested an integrated approach that incorporates variables such as the ventricular function, aortic size, pulmonary pressures, arrhythmias and saturations, as well as the parameters achieved on CPET to use as guidance to prescribe individualised exercise plans for ACHD patients.46

    Training protocols

    The majority of published exercise training programmes published in ACHD patients focus on a HR-driven protocol while allowing patients to determine the type of physical activity to achieve the HR goals. Duppen et al randomised ToF and Fontan patients to a three times a week exercise programme or no exercise intervention.47 The exercise programme included 40 min of aerobic activity at a HR goal based on the patient’s HR reserve, determined from a CPET. There was no decline in cardiac function or significant arrhythmias, and for the systemic RV patients, an improvement in peak VO2 or stroke volume was observed.44 Of note, as resting HR may vary according to the time of day and other factors, using % of the predicted HR or peak HR may prove more accurate goals in creating exercise programmes. For those who are severely limited or high risk, enrolment in a cardiac fitness/rehab programme may be beneficial.

    Considerations in specific populations

    Respiratory function continues to be a key component in exercise capacity and correlates with outcomes.36 48 49 Given their reliance on the muscle pump and to a lesser extent the ventilator pump, Fontan patients may disproportionately benefit from skeletal and inspiratory muscle training, and there is growing interest in inspiratory muscle training to improve ventilator efficiency during exercise in these patients.50

    Patients who are pacemaker dependent or have ICDs in place may benefit from further assessment as to the pacemaker/ICD settings, specifically regarding maximal tracking HRs and programming to avoid inappropriate shocks. For those who are pacemaker dependent, HR goals can be less useful in the exercise prescription as they do not reflect the patient’s physiological state. For these patients, we recommend use of a rated perceived exertion (eg, Borg) scale. Using the scale value selected by the patient as a correlate for a typical HR response heart (Borg/rate perceived exertion 12~120 bpm) the provider can create a prescription based on expected values. However, patients will need to have a clear understanding of the use and limits of the scale.

    Figure 4 provides a summary of our recommended approach to exercise reference for ACHD patients. As providers discuss exercise programmes, it is important to have frequent communication and discussions regarding warning signs (lightheadedness, presyncope, palpitations and so on) that may require further evaluation. ‘Specific diagnosis’ refers to the diagnosis of the exercise limiting factor.

    Figure 4
    Figure 4

    Suggested algorithmic approach to consulting sports participation for ACHD patients. ACHD, adult congenital heart disease; CPET, cardiopulmonary exercise testing; HR, heart rate; HRR, heart rate reserve; ICD, implantable cardioverter defibrillator; VO2, oxygen consumption.

    Conclusions

    Exercise testing and sports participation play key roles in the regular follow-up and treatment protocols for ACHD patients. Routine objective assessment of exercise capacity via CPET provides important information on cardiac function, possible extracardiac aetiologies of exercise limitation, as well as guidance for exercise prescription. The benefits associated with routine exercise for ACHD patients parallel those for other adult populations with and without cardiac conditions without generalised associated with higher risks for ventricular arrhythmias, sudden death or heart failure exacerbation. Counselling on participation in competitive sports activities should be individualised based on available guidelines.

    Key messages

    • Adult congenital heart disease (ACHD) patients have decreased exercise capacity as compared with healthy peers, regardless of the underlying lesion and repaired status. A nearly direct correlation exists between the severity of the congenital lesion and the extent of exercise intolerance across the spectrum of ACHD.

    • Objective assessment of exercise capacity with a cardiopulmonary exercise testing (CPET) constitutes an integral part of routine follow-up for ACHD patients. Importantly, it provides relevant information on cardiac and extracardiac causes of exercise intolerance as well as prognostic information and, in certain instances, assistance in guiding therapy.

    • Different anatomic lesions may show distinctive patterns on CPETs, and the various parameters, including the peak oxygen consumption, minute ventilation/carbon dioxide production slope and the ventilatory threshold, should be interpreted in the context of the specific lesion.

    • Routine exercise was not shown to be associated with excess risk of sudden cardiac death or decline in systemic ventricular function among ACHD patients.

    • Rather, an improvement in both cardiac function and quality of life is expected for ACHD patients for exercise and sports participation. Thus, sports restriction and limitation of exercise should be avoided if possible.

    • Specific guidelines for sports participation for ACHD patients, with relevant restrictions for higher-risk lesions, are available, and should be used to guide providers in prescribing exercise to this patient population.

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    Education in Heart articles are accredited for CME by various providers. To answer the accompanying multiple choice questions (MCQs) and obtain your credits, click on the ‘Take the Test’ link on the online version of the article. The MCQs are hosted on BMJ Learning. All users must complete a one-time registration on BMJ Learning and subsequently log in on every visit using their username and password to access modules and their CME record. Accreditation is only valid for 2 years from the date of publication. Printable CME certificates are available to users that achieve the minimum pass mark.

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    Footnotes

    • Contributors Both YB and KS contributed to the planning, conduct and reporting of the work described in the article.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Commissioned; externally peer reviewed.

    • Patient consent for publication Not required.